Getting Kicked in the Ass is a Good Reason to Come Back

EDIT:  I was (and still am) incredibly frustrated when I wrote this post.  I had in mind to edit its contents, but angry or not, the words are honest.  I have been chronically unwell for a long time now and sometimes, it gets really difficult to smile.  And then I think about what someone once told me on this site several years ago and... Well, darn it, I'm going to try and smile anyways and "Be positive" even though I don't have B+ blood type.  Sorry for the anger; it isn't towards anyone at all.

---

I'm angry.

In general, having thalassemia sucks.

But what sucks even more is when you are shuffled around, pushed around, and then marginalized by doctors, healthcare workers, medical insurance companies, and even the federal government who can't agree on what's going on much less how to treat anything because you have alpha thalassemia instead of beta thalassemia.

In some ways, I wish I had beta thal.  If anyone understands anything about thalassemia, it has to do with beta thal.  If anyone understands any sort of treatment for thalassemia, it's treatment for beta thals. 

The minute I say I have Hemoglobin H Disease - a type of alpha thalassemia - one of two things invariably ends up happening.  Most commonly and most frustrating of all is, nothing gets taken seriously and the dominoes fall from there.

Symptoms get brushed aside or attributed to things completely unrelated and go untreated... 

You exhaust yourself at school and work because you start to believe that maybe you really are just imagining everything...  Hey, you aren't dying; things can't be that bad, right?  Yeap.

You get sick and keep getting sick, but whatever... Life goes on; deal with it.

You're exhausted, you're aching in your organs, you're aching in your head, you're aching in your lower back, your heart pounds, you feel dizzy, you have cold sweats, your urine never stays clear of that tinge of brown no matter how much water you drink... But hey, you just need to take a break is all!

You can't hold a job for any length of time because you're way too damned tired all the time and spend half of your time being sick or being in chronic pain... Hey, that's just stress - relax!

You can't earn enough money to pay for all the bills that keep accumulating and you look at the bills and wonder if they were even worth it.


At some point, you get the courage to seek help again because you think maybe this time, it will be different, but then once it's all done with, you realize you're back at the beginning again struggling to experience a higher quality of life with a hemoglobin level of 8 and with no treatment plan in sight or social services to help you.

Because you aren't beta thal.  Because what they think and understand isn't what you are.  Because even when all the blood and specimen tests come back and say otherwise, they still think that you should be okay.  Because you only have Hemoglobin H, you SHOULD be fine, you shouldn't need transfusions, you shouldn't be so tired, you shouldn't be in so much pain in places that have no other reason to be hurting, you shouldn't be pissing so much bilirubin, your teeth shouldn't be dying all on their own for no good reason, and you should be able to function like a perfectly healthy, normal human being.  Everyone thinks you SHOULD be fine, and so you SHOULD be fine and then you end up being 'fine'.


Well, since nobody wants to take it seriously, neither will I.

So here I am, an anonymous Hb H patient, and I am perfectly and absolutely FINE.

Hi HbH,

Have you ever tried to find help through the Cooley's Anemia Foundation or been to any of the thalassemia centers of excellence in the US?

I hate to say this, but I have been to CHO and did a consultation and additional lab workup and such there - multiple times actually... and to no avail.

The things I have ever reported to them came back to me as simply a "part of [having thalassemia]" and something to simply deal with.

My physician keeps encouraging to see a thalassemia specialist again because he's seen my records and he's seen me come into his office sick and in pain for reasons only thalassemia can explain (in his experience and to his understanding), but the previous times I have gone to CHO to get things looked at were NOT encouraging at all.

Considering the reputation CHO has, I really don't know where else to turn to should I need the help.

I'm probably not going to say anything you haven't heard before. Accepted practice is to not transfuse at Hb of 8. The risks from iron, antibody reactions, and even a minute chance of acquiring disease outweigh the benefits of transfusion. Other than low Hb you should experience only mild symptoms...and so on. HbH is often compared to non-transfusing beta intermedia. Take folic acid. Have a good day.

Well, it's nice to have standards and measurements of Hb and analysis of genetic mutations and what your specific mutation should mean, but does anyone try to measure the true quality of life of patients or all patients lumped together by mutation and Hb level and assumed that they are all the same? And as more patients are observed over time, is there any adjustment made to prior notions of what to expect in an individual patient? And is there ever any consideration that people are individuals and combinations of certain traits in a patient might lead to a lower quality of life? And if HbH is similar to non-transfusing beta intermedias, should more consideration to transfuse be given as patients get older, as is the case with beta intermedia? And shouldn't the doctors be asking some of these questions?

When it comes down to it, there aren't many options with HbH. Folic acid. A good diet and a good multivitamin. Live with it. Or make a decision that you want to experience life with a normal Hb level and pursue a goal of transfusing, and see if you can get your doctor to agree to at least occasional transfusions. I will say that I have heard from adult beta intermedias who started to transfuse later in life and once they did, wished they had started a lot sooner, because their quality of life was so improved. I do think it's a big decision and acceptance of chelation will have to be a given. I also wonder if it would help your case if your doctor was to write to Dr Vichinsky at CHO and describe your physical condition when you've been examined and ask if the deterioration in health would indicate a need for a new evaluation and if transfusions might be indicated even with an Hb of 8.

I don't know how much of this list you've seen before but we have a list of things to avoid with alpha thal. I also want to mention that I am noting more and more references to beans and legumes in general as foods some alpha thals should avoid, and not just fava beans. It might be worth it to try avoiding any type of beans and products containing any bean or legume, although with the amount of soy used in foods this may be difficult, for a month or so, to see if there is any noticeable difference.
http://www.thalassemiapatientsandfriends.com/index.php?topic=3410.msg34830#msg34830

I'm probably not going to say anything you haven't heard before. Accepted practice is to not transfuse at Hb of 8. The risks from iron, antibody reactions, and even a minute chance of acquiring disease outweigh the benefits of transfusion. Other than low Hb you should experience only mild symptoms...and so on. HbH is often compared to non-transfusing beta intermedia. Take folic acid. Have a good day.

Hi Andy, regardless if I might have heard some or all of this before or not, I really appreciate hearing from you anyhow and seeing how other patients with varying degrees of thalassemia have coped and have managed to make their decisions regarding transfusion therapy.

Yes, I have always been told the same things and I myself fully understand the risks involved and this was a major reason I never did any regular transfusions growing up.  My own (and my family's) concerns about the risks along with what the doctors kept insisting (you should be fine without, transfusions are very risky, etc) was enough to scare me and my family away from them.

Take folic acid every day and have a good day IS exactly the response I have been given for all of my life, regardless of how I have felt.

Well, it's nice to have standards and measurements of Hb and analysis of genetic mutations and what your specific mutation should mean, but does anyone try to measure the true quality of life of patients or all patients lumped together by mutation and Hb level and assumed that they are all the same? And as more patients are observed over time, is there any adjustment made to prior notions of what to expect in an individual patient? And is there ever any consideration that people are individuals and combinations of certain traits in a patient might lead to a lower quality of life? And if HbH is similar to non-transfusing beta intermedias, should more consideration to transfuse be given as patients get older, as is the case with beta intermedia? And shouldn't the doctors be asking some of these questions?

Yes, yes, a thousand times yes.

Where I do not believe in transfusion therapy or any other thal-related therapy being taken lightly (they can be VERY life-altering decisions which I fully respect), I believe that patients are all individuals and that nobody nowhere is going to have the exact same experiences with thal and that the medical profession needs to be more open minded about evaluating these experiences.

My own experiences with HbH have been more severe than most according to the hematologists I have seen in the past, but they are also more inclined to attribute the symptoms to things other than thal and I suspect that is because my symptoms are falling outside of what is considered "normal".  There is resistance, in my opinion and experience, to accept that there is a possibility of varying degrees of anemia and anemia-related problems for certain types of anemias - alpha thal, HbH being one of them and quite possibly/probably beta thalassemia intermedia.

My own primary care doctor goes by the patient and not necessarily by the "rules of thumb" and so has kept track of my health in lots of detail.  He knows what he sees, but he is not a hematologist and admits to such and so prefers to defer advice to a hematologist and that has been where my frustrations have really come to fruit.

When it comes down to it, there aren't many options with HbH. Folic acid. A good diet and a good multivitamin. Live with it. Or make a decision that you want to experience life with a normal Hb level and pursue a goal of transfusing, and see if you can get your doctor to agree to at least occasional transfusions. I will say that I have heard from adult beta intermedias who started to transfuse later in life and once they did, wished they had started a lot sooner, because their quality of life was so improved. I do think it's a big decision and acceptance of chelation will have to be a given. I also wonder if it would help your case if your doctor was to write to Dr Vichinsky at CHO and describe your physical condition when you've been examined and ask if the deterioration in health would indicate a need for a new evaluation and if transfusions might be indicated even with an Hb of 8.

Everyone says to choose the path of safety, the path of least resistance - avoid getting transfused and avoid the dangers and risks that come with regular transfusion therapy - and I have, for nearly 30 years now... but especially in the last 5 years, it just gets worse and worse and worse.

After eating well, taking all my supplements and then some, avoiding all the oxidative foods and then some, trying to take care of myself and not over exhaust myself all of my life, my health and quality of life has overall started deteriorating over the years.

It's a big decision - a potentially life changing decision - when it comes to transfusion therapy and I know it is and I would avoid it if possible... but I don't think avoidance is the answer anymore.

Not if I want to become fully independent.  Not if I want to be able to function more productively in my life and not constantly worry about getting sick and then having to sit out for weeks at a time to let my body recuperate.

Unfortunately... "do not transfuse" is still the answer that is being pushed along with "you're not taking care of yourself", "your problems must have some other reason" etc and I feel invalidated and even stupid at times for even considering such an option.

Because I only have HbH and shouldn't be having these problems that people with more severe forms of thal have.

But I will take your advice and ask my doctor and see if he will write me that letter to Dr. Vichinsky.

When I went to CHO, I wasn't seen by Dr. Vichinsky even though I was seen in their Thalassemia Center.  The multiple times I went, I was always deferred to another doctor and to even their nurse practitioners even though my appointment was for Dr. Vichinsky.

... Do you think being seen by Dr. Vichinsky himself will help or at least help me find answers and help me in trying to make a life-altering decision or is it better to not hope and just "wait and see"?

I don't know how much of this list you've seen before but we have a list of things to avoid with alpha thal. I also want to mention that I am noting more and more references to beans and legumes in general as foods some alpha thals should avoid, and not just fava beans. It might be worth it to try avoiding any type of beans and products containing any bean or legume, although with the amount of soy used in foods this may be difficult, for a month or so, to see if there is any noticeable difference.
http://www.thalassemiapatientsandfriends.com/index.php?topic=3410.msg34830#msg34830

Funny you should mention this; legumes - including peanuts - oftentimes give me unexplained side pains where my spleen is.

I will look at the list; thank you for providing it and thank you for your thoughts in general.

If you do have another visit to CHO, I would specifically ask to see Dr Vichinsky. I don't know if there is anyone on earth as knowledgeable about alpha thal as Vichinsky. From my experience talking to him at conferences, I would suggest you come prepared with a list of questions. Vichinsky is an incredibly busy man with people pulling at him from all sides at all times, so an organized approach is best when talking to him. Have all your questions listed and well organized, along with any possible follow up questions. I have observed that he has an uncanny ability to quickly analyze a situation and not only get down to the physiology but also the psychology of the person involved, seeing the person as a whole, rather than separate parts. I do not know if he would recommend transfusion, but I do have faith in his knowledge as to what the correct course to pursue will be. If your doctor could write directly to Vichinsky in advance so that he has some prior understanding of the true status of your day to day health and quality of life, that this may help, as the words will be coming from a doctor that sees you regularly.

During my internet searches on different topics I come across many observations, and that is why I mentioned legumes in general. What I encounter suggests that more than fava beans should be considered as possible causes of hemolysis in alpha thals, and possibly also in beta thals. There may be even more foods and substances that can cause hemolsysis and I often wonder about high amounts of garlic because it is well known that it causes hemolytic crises in dogs. Doing trials on your own to see if stopping certain foods for awhile improves any aspect of your health...reduction of a swollen spleen for example or any obvious change in Hb...is worth a try to see if any outside influence are affecting your health, compounding the HbH.

The one major difference between alpha and beta thal is there is no other hemoglobin to fall back on, as is the case with the potential role of fetal hemoglobin in beta. This does leave little in the way of options and transfusion has long been thought of as an absolutely last resort. However, quality of life HAS to enter into the equation, and with modern chelation, ideas have changed and continue to change. I know you would feel better with transfusions. But both you and the doctor have to agree that your quality of life is suffering so much, that transfusion is the only real option that will help you carry on a normal life. It is a big decision and only you can really decide if you've had enough living with serious anemia. Your inability to lead a life of decent quality should be stressed greatly if you do meet with Vichinsky. I lived with an Hb of 8 for a short period after losing a lot of blood during surgery, and frankly, I could barely function. I spent most of my time in bed. This one instance in my life created a great deal of empathy for those who always live like that. I think it must take a strong will to accomplish even the most basic things and this strength I have seen in patients has had a great deal to do with why I have chosen to continue my work in this group long after my best friend has departed us. I think thals are one of the biggest classes of the ignored on earth and I cannot ignore that fact. With hundreds of millions of carriers on earth, there should be a much greater effort to help those in need rather than shuffling them off unless they are transfusing. I feel that for too long, the problems of intermedias and minors have been downplayed and if it is to change, it will take much effort to alter the preconceived notions about minor and intermedia. That effort has to come from patients and families and every advocate for thals. We have to change these preconceived perceptions if people are going to get the help and attention they need. To every patient: When a doctor says that someone with your status should not have symptoms, please speak up and say "well doc, I DO have these symptoms and they are not all in my head." Patients insisting on help and insisting on more research into the non-major types of thal have to get their voices heard.

Andy,

I can't thank you enough for both the time and energy you have put into your thoughts and words - not just in response to me, but in response to everyone here.

I have recently seen my primary care physician and he is referring me out (again) to CHO and specifically to Dr. Vichinsky if at all possible and I am crossing my fingers and hoping for the best.

My PCP is a compassionate man and just as importantly (or even more importantly depending on how one sees it) a very reasonable and knowledgeable one.  He said a similar thing about functioning and about quality of life and that he was unsure how I have thus managed to function as I do with my low levels of hemoglobin.  I have little doubt that the topic of quality of life (which was why I went in to see him) will come up in the referral.

Related to that, we talked about ways to improve my symptoms (by improving hemoglobin) and he is also urging me to seriously consider having a splenectomy done to minimize my RBCs being prematurely filtered out.  Remove the spleen = give the RBCs a little longer to live which will (hopefully) improve my hemoglobin levels.  Of course as a trade-off, there is the risk that removal of the spleen might = more immune problems later.

In regards to transfusions, he said that because my RBCs have a rather short/truncated lifespan owing to the Hemoglobin H, he is concerned about where a normal individual might experience a substantial boost in hemoglobin for two to three months, I would only experience a slight improvement for a few weeks which would mean that I would quite possibly need to then do transfusions regularly to achieve any sort of longer term improvement.  

Of course, this then goes into the topic of chronic transfusions vs intermittent as-needed transfusions which presents its own list of things to consider and weigh which I am more than willing to consider and am currently considering.

It's... kind of funny.

My doctor is not the sort who likes prescribing medications or procedures just for the hell of it.  He is a very straightforward man (thank goodness!) and tells things like it is and if he thinks a medication is unneeded or a procedure is unwarranted, he will tell it like it is and explain his reasoning why.  He has always been conservative about dispensing medication while being liberal about his thoughts and yet never hesitates to prescribe and treat when he sees that the situation is necessary.

And... yet.

In that one visit, I got told to seriously consider a splenectomy, was given a referral out to CHO, and got prescribed a psychostimulant (with very explicit directions and warnings) as a very last resort energy booster should I need it to help me make it through work and life in general while I struggle with lagging energy levels and wait for a 'better' solution.

Oh well.

What will be... will be.

Hello HbH,

I sympathize with what you are going through and hope things get better. I was in the same boat for the longest having pains and fatigue at school and at work. Spending entire nights in the hospital just to be told its just an episode and being sent back home. Still to this day I will be so tired sometimes that it feels like I've passed out instead of dozed off. I would like to ask if exercise on a regular basis. This helped me tremedously. I feel better and don't have the episodes of stomach pains any more. I am a beta thal intermedia and maintain a 8.2 to 9.0Hgb count. The Dr. also recommended removing my spleen if it gets any larger, but I don't see that happening. I'm not sure what a pyschostimulant is but I've found that sometimes an energy drink in morning gets me to where I get enough momentum to make it thru the day.

I hope you get better and maybe something I've said has helped. Take care of yourself.

Hey there, Bobby,

Thank you so much for your thoughts!

Yes, exercise is a fairly regular and important factor in my lifestyle.  I used to lift weights regularly until my exhaustion levels made it really difficult to maintain a more regular come-and-go routine at the gym.  I still go to the gym when I can, but exhaustion and being easily exhausted has definitely become a major setback in keeping up with a routine.  I take walks when I can otherwise and go rollerblading (running and jogging puts too much oxygen-deficiency pressure on me, regardless of how 'healthy' I am otherwise) when the notion strikes.

Thalassemia and thalassemia-related problems aside, I am otherwise a very healthy person.  I maintain a good weight (usually), have solid muscle tone, eat well (which includes NOT following the FDA guidelines; if I followed them, I would probably be malnourished and diabetic by now) and eat fairly clean, and take my supplements pretty faithfully. 

It's kind of frustrating that despite the fact that I am an otherwise healthy person, my thalassemia issues like to come in and kick my butt - sort of like reminding me that, "Hi!  I'm still here!"  Having the on-again, off-again fairly regular pains and aches and exhaustion just stinks sometimes when you're trying to take care of things in life.


May I ask you a few questions?

I have not met anyone (not including other people here who also share a similar Hb level whom I haven't met yet, of course ) who maintains a Hb level similar to mine and am interested to see how your experiences have been.

If I am being too nosy, please let me know and by all means, you most certainly don't have to answer all these questions if you don't want to.


1)  At your current Hb levels, do you transfuse?  What has your transfusion history been like?

2)  What have your Hb levels been like?

3)  Do you have any advice for making it through work?  With the fatigue issues, especially, how have you managed?

4)  Did you experience any other thalassemia-related issues while maintaining the Hb levels you're at now and if so, how were they dealt with?

5)  How are your ferritin levels?  Do you do chelation?


For myself, I have always maintained a Hb of 8 (8.6 being the highest, 7 when I get sick or am really stressed/my body is really stressed), have had fluctuating levels of iron up to 2.5x the normal amount (the doctor says I need to get a more accurate ferritin count since blood levels can be misleading, but 2.5x the normal is still 'okay' for me, apparently), have been transfused three different times in my adulthood, and have always struggled with illness and fatigue issues when they strike.

On the other hand, I also noticed that I seem to build muscle faster than others do.

Thanks again for your thoughts; I really appreciate them!

Hi HbH,

I'm glad to answer any questions you have. I don't get to talk about my thal much, due to the fact most people in Alabama have no idea what it is and the only other person I've ever met with it was my younger sister. She a minor and only suffer from pain and fatigue sometimes.

1)  At your current Hb levels, do you transfuse?  What has your transfusion history been like?

I do not transfuse. I had maybe 6 or 7 transfusions until the age of 13, then I stopped completely. When I turned 30 feelings of weakness and lethargy caused me to go to the Dr. and my Hb was a 6.9 so I had a transfusion then. I am 34 now.

2)  What have your Hb levels been like?

All my life they've been between 7-8.5. I once had one recorded at 9.1 after I started taking a supplement called N.O Explode for building muscle.

3)  Do you have any advice for making it through work?  With the fatigue issues, especially, how have you managed?

My best friend for staying alert at work is early morning caffeine. I used to drink coffee, but didn't feel that it helped much until one day I tried the NO Explode(It contains a high amount of caffeine) one morning before leaving the house. I was a whole different person. I was also taking a serving before lunch, which is when I go to the gym and for weeks this routine had me alert and energetic during work. I'm not taking it right now because they reccomend a cycling period and I can tell the difference in the a.m and after working out. Energy drinks work ok to, but I'm not a fan. I'm normally a high energy kind of person, but when I crash it's seems to last for weeks at a time and it's tough to rebound from.

4)  Did you experience any other thalassemia-related issues while maintaining the Hb levels you're at now and if so, how were they dealt with?

I don't have any major issue or pains, just small things that I kinda disregard. I used to have these episodes that Dr's called "Crisis's" where my stomach would cramp around my spleen area about once every 1-2months. It was awful pain. I would have to sleep in an upright position and take sleeping pills to get some rest until it passed. I had Cat Scans, and numerous other test and no Dr's could tell me what caused it. That doesn't happen any more. I'm not sure why, it could be the 8.5+Hb levels, but I honestly don't know. I'm having some slight joint pains from time to time, could be cause by my level of activity though. I played basketball alot and I play softball sometimes. I still have yellow jaundiced eyes from childhood, but other than that no one could tell I had Thal or anything else. I also found that removing sugar from my diet make my stomach less irritable.

5)  How are your ferritin levels?  Do you do chelation?

I recently just finished a course of Exjade. I was on it for 5mths and my ferritin went from around 1600 to under 800 and the Dr. took me off of it. I drink alot of tea. Mostly green and black teas is hopes that I don't absorb any iron from the mass amounts of food I consume. Oddly enough the more muscle mass I add the better I feel. Unlike you though I struggle to build muscle. I've gained 20lbs over the last few years, but seem to be stuck at 180lbs. I made it to 190lb this summer, but dropped back to 180lb after removing sugar from my diet. I am 6'2.8", which at that height is thin.

Please feel free to ask any other questions you have. You can email me at Lanisb@Gmail.com and my facebook link should be under my profile info.

Dear HbH,

I dont know what to say....I cant say that I empathise, because I wouldnt know what you are going through...

I can only hope that all of us friends at thalpal are of some help, Andy of course is always a great source of knowledge, support and strength....

I can only pray that things are better for you and you are happy always....

Lots of love,
Madhavi

@ Bobby:

Thank you so much for your thoughts.

The pains you describe are like the pains I have experienced as well.  They come every other month or so and they last anywhere from two weeks to a whole month.  They are where my spleen is and sometimes 'deeper' - towards my left side starting from the sternum and inwards and spreading out to my side - and the pain is always sharp and lingering and constant.

I too did the whole CAT scan bit and even went through an upper endoscopy as the GI specialist I ended up getting referred to thought I might have an ulcer.  Everything came back clean and my primary care doctor who I ended up switching to during this time (who is my current doctor now) was the one who suggested that these pains were/are simply thalassemia-related - perhaps a reaction to too much work, stress, and/or oxidants my body is undergoing and processing.

Too much exercise and exhaustion can lead to excruciating lower back pain and excess bilirubin where it shouldn't be which my doctor has said is part of my body reacting to being pushed too hard.

I will (hopefully) be seeing a hematologist again soon and will hopefully be able to sit down and hash things out. Your answers to my questions are things I will be considering when I speak to the hematologist so I really appreciate your thoughts.

@ Madhavi

Thank you for your well wishes; I really appreciate them. 

Too much exercise and exhaustion can lead to excruciating lower back pain and excess bilirubin where it shouldn't be which my doctor has said is part of my body reacting to being pushed too hard.

I may have experienced this last night. I was helping a friend coach his 13yrs old boys basketball team and after a few trips up and down the court I got bad lower back pains. They weren't unbearable, but it was like my lower back was really tight and it was painful to stand straight. After I stopped it went away, but it made me think of this. As I sit at my desk now I have a few muscle aches, but other than that I feel pretty good.

Hey Bobby,

I hope you and your family had a great Thanksgiving; I'm sorry I was unable to respond to your post until now. 

The pain you describe is definitely similar to what I have experienced after a certain amount of physical exertion.

For me, there is a direct correlation between how fatigued I am and how much pain I end up experiencing; the greater the fatigue, the more intense the pain.  Additionally, my urine at those points - regardless it seems of how much I drank before - gains a darker brownish coloration to it.  I once went to my doctor after such an incident and he said that it seemed that I was having an 'episode' in regards to my thalassemia and when he ordered a urinalysis, the results showed greater amounts of bilirubin in it which to him, confirmed his diagnosis of me experiencing a thalassemic episode.

Walking a mile won't trigger the pain or the over exhaustion, but being forced to run - or try to run - a mile, walking around for a period of time without any rest breaks greater than 2 - 3 hours, and simply doing too much physically intensive work when I am already kind of tired will trigger the pain episode.

When I was younger, school on its own without PE was okay for the first few hours as I was sitting for most of the time.  PE class would almost invariably exhaust me and I would end up falling asleep in my next class or be so fatigued that I would be spaced out like a zombie.

When I got my first job at age 17 that required me to do a lot of walking around and light manual labor for hours on end back to back, I started really noticing the effects of the exhaustion - namely, the pains in my back.

When I was going through university starting at age 18, I started experiencing the pains on a more frequent and regular basis - even when I didn't think I was doing so much work - as I was forced to run around between all my classes and then also juggle work at the same time.  During this same time, I also got sick almost every other month.

Nowadays, my biggest concern is being able to hold out against this pain for the sake of work which, once again, requires a lot of running around and light manual labor.  Obviously, the best option is to not trigger it and/or deal with the cause of the trigger (like over exhaustion), but if you can't cure what is creating the problem, then you simply have to DEAL with the problem.

I have an appointment @ CHO @ December 9; I am hopeful that Dr. Vichinsky can offer me some thoughts, options, and/or solutions to this and ideas on how to manage my thalassemia that seems to be worsening as I get further into my adulthood.

On the other hand, I am also scared that I will be disappointed.

I'm not looking for a cure and I am not even looking for definitive answers or problem solving, but I need help and I need viable options because I just can't manage all of this on my own and alone and without a proper medical professional to guide me.

HbH,

Have you ever kept a food diary to see if you can see any connection between certain foods you've eaten and the hemolytic episodes? Our member, Jan has confirmed that certain foods do send her hemoglobin level downward. She is currently waiting for test results to see if alpha thal may be involved, but regardless if it is alpha thal causing the hemolytic crisis, the same foods and chemicals can cause these sudden drops in Hb in several different conditions, including alpha thal. Fava beans are the most well known food offender, but other beans and legumes can have that same reaction in some people. If you are certain you're not having any occasional chemical exposure to things like moth balls or gasoline fumes, keeping a diary of what foods are ingested may be enlightening. One problem, of course, is not knowing when legume products are added to foods. Soy seems to be in everything these days. So it would take some work to keep track of everything eaten.

I'm glad you're going to see Dr Vichinsky. Make a point of asking him what you can do for yourself to improve the quality of your life. This is actually something doctors need to hear more often from patients. I really do believe that doctors in general, have little idea what the true quality of life is for many patients, and reminding them that this really is the most important issue is essential.